scholarly journals Dramatic Intracerebral Hemorrhagic Presentations of Reversible Cerebral Vasoconstriction Syndrome: Three Cases and a Literature Review

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Joel M. Stary ◽  
Bonnie H. Wang ◽  
Seong-Jin Moon ◽  
Huan Wang

Reversible cerebral vasoconstriction syndrome (RCVS) refers to a number of disorders characterized by severe and sudden-onset (“thunderclap”) headaches and angiographic features of reversible, segmental, multifocal vasoconstriction of cerebral arteries. Although RCVS generally resolves without significant sequelae, a rare and possibly underrecognized hemorrhagic presentation has a worse potential course. We report three cases of hemorrhagic RCVS and review the literature. Three females (42, 54, and 33 years old, resp.) presented with severe headache, neurological deficits, and dramatic intracerebral hemorrhage (ICH). Patient 1 presented comatose with a 9 × 4 × 6.6 cm left deep intraparenchymal hemorrhage (IPH) and 1 cm midline shift. She underwent emergent surgical intervention. Patient 2 had a 3.3 × 1.5 cm left superior frontal IPH that enlarged to 4 × 2.5 cm within 12 hours with worsening headache and neurological deficits. She was successfully managed nonoperatively. Patient 3, after uncomplicated pregnancy and delivery, presented with a 1.5 cm left superior parietal IPH on postpartum day 7. Two days later, she acutely developed right hemiplegia. Repeat CT demonstrated a new 3.3 × 1.7 cm left frontal IPH. She was also successfully managed nonoperatively. Many diverse conditions are grouped within the category of RCVS. Dramatic ICH remains a rare and possibly underrecognized presenting feature. Prompt diagnosis and management are essential for obtaining the best outcome.

Cephalalgia ◽  
2009 ◽  
Vol 30 (1) ◽  
pp. 42-45 ◽  
Author(s):  
Y Soo ◽  
AB Singhal ◽  
T Leung ◽  
S Yu ◽  
H Mak ◽  
...  

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset recurrent ‘thunderclap’ headaches with reversible multifocal narrowing of the cerebral arteries, often associated with focal neurological deficits from ischaemic or haemorrhagic stroke. It has been associated with exposure to vasoconstrictive drugs, pregnancy, migraine, and a variety of other conditions. Whereas the pathophysiology of RCVS remains unclear, changes in the levels of female hormones are considered important because RCVS predominantly affects women and is frequently associated with pregnancy. We report a patient with angiographically confirmed RCVS whose MRI showed reversible brain oedema, suggesting an overlap between RCVS and the reversible posterior leucoencephalopathy syndrome. The only identified risk factor was oral contraceptive pills started 1 month prior to onset, supporting a role for female reproductive hormones in precipitating this overlap syndrome.


Author(s):  
Neelu Desai ◽  
Rahul Badheka ◽  
Nitin Shah ◽  
Vrajesh Udani

AbstractReversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.


Author(s):  
Héctor Montenegro-Rosales ◽  
Blanca Karina González-Alonso ◽  
Omar Cárdenas-Sáenz ◽  
Alonso Gutierrez-Romero

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is defined as a clinical and radiological syndrome that comprises a group of disorders characterized by sudden-onset severe headache and segmental vasoconstriction of the cerebral arteries with resolution within 3 months. Case presentation: A 51-year-old female patient with a 2-week history of sudden-onset severe headache, visual disturbances and cerebellum; no relevant imaging findings, except for an infundibular dilation at the origin of the posterior communicating artery, and so, angiography was performed. When symptoms persisted, a new imaging study was carried out with findings of RCVS as the cause of the symptoms from the beginning. Conclusions: Findings of RCVS can be obtained in various vasculopathies of the nervous system and vasculitis, being misdiagnosed, and so, clinical suspicion is essential; if vasoconstriction is not demonstrated on the initial image and other diagnoses have been excluded, the patient should be managed as having possible or probable RCVS.


Cephalalgia ◽  
2015 ◽  
Vol 36 (3) ◽  
pp. 289-293 ◽  
Author(s):  
Makoto Kobayashi

Background Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches with reversible cerebral vasoconstriction, and often precipitated by the postpartum state and vasoactive medications. We describe a case of a patient with RCVS induced by amezinium metilsulfate, a sympathomimetic drug, in whom magnetic resonance angiography (MRA) initially revealed diffusely dilated cerebral arteries. Case description A 34-year-old woman was prescribed amezinium metilsulfate for hypotension. Twelve days later, she suffered from abrupt severe headaches and was referred to our department. She had no neurological deficits; however, MRA revealed diffusely dilated anterior, middle, and posterior cerebral arteries with vasoconstriction. She was tentatively diagnosed with RCVS and successfully treated with verapamil for headache. Nevertheless, follow-up MRAs disclosed widespread segmental vasoconstriction that resolved in two months. Discussion Diffuse cerebrovascular dilation has not been addressed but may be associated with RCVS pathophysiology. In addition, physicians should bear in mind that amezinium metilsulfate can potentially induce RCVS.


2019 ◽  
Vol 34 (6) ◽  
pp. 944-944
Author(s):  
E Schultz ◽  
S DeDios-Stern ◽  
G Ovsiew ◽  
N Pliskin

Abstract Objective Reversible cerebral vasoconstriction syndrome (RCVS), a relatively rare condition presenting as severe thunderclap headache with or without neurological deficits, is characterized by vasoconstriction of cerebral arteries which often resolves within three months and may be associated with stroke. Previous case reports examining neuropsychological profiles of RCVS patients demonstrated stroke-specific and/or diffuse global impairments that often exceed impairment expected post-acute phase of clinical stroke and possibly suggest residual deficits related to RCVS alone (Perdices & Herkes, 2018). Method The present case is a 36-year-old right-handed African-American male who sustained a left thalamic hemorrhage with extension into the ventricular system following marijuana use. Cerebral angiogram was suggestive of RCVS. The Montreal Cognitive Assessment (MoCA), Western Aphasia Battery (WAB), Repeatable Battery for the Assessment of Neuropsychological Status- Update (RBANS), and Peabody Picture Vocabulary Teat- 4th edition (PPVT-4) were administered at bedside. Results Neuropsychological testing was abnormal with severe global impairments on tasks requiring attention and processing speed, visuospatial abilities, immediate and delayed memory, and executive functioning. The patient’s expressive speech was characterized by fluent output with pronounced provoked and unprovoked phonemic and semantic paraphasias. Additionally, he evidenced confabulatory responses during memory testing and lost mental set during tasks requiring simultaneous attention. Conclusions Considering the patients clear sensorium at the time of the evaluation, these deficits extend beyond what would be expected by a single thalamic lesion and implicate more generalized cerebral dysfunction secondary to his RCVS. Consistent with previous case studies, this case demonstrates how RCVS may confound and contribute to neurocognitive deficits even after hemorrhagic stroke.


Author(s):  
Philip Gerretsen ◽  
Ralph Z. Kern

Background:Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and focal neurological deficits, often without a clear precipitant. RCVS may be mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the presence of similar angiographic features of segmental narrowing of cerebral arteries. We discuss the clinical features of a young female migraine patient who developed TCH and was found to have RCVS following initial treatment with corticosteroids for PACNS, in the context of a systematic review of the available medical literature.Methods:A Medline™ search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy.Results:Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is characterized by sudden, severe headache at onset, normal CSF analysis, vasoconstriction involving the Circle of Willis and its immediate branches, and angiographic or TCD ultrasound evidence of near-to-complete vasospastic resolution within 1-4 weeks. It occurs typically in the context of vasoconstrictive drug use, the peripartum period, bathing, and physical exertion.Conclusion:Initial and follow-up (within 4 weeks) non-invasive angiographic studies are indicated in patients who present with TCH or who have clinical presentations that could be consistent with RCVS or PACNS in the absence of a well-recognized secondary cause, such as subarachnoid haemorrhage. Early reversibility of cerebral vasospasm is the key neuroradiological feature that supports the clinical diagnosis of RCVS.


2021 ◽  
Vol 16 (1) ◽  
pp. 78-82
Author(s):  
Paul J Alapatt ◽  
Ajay Panwar ◽  
Gigy Varkey Kuruttukulam ◽  
Kaushik Sundar

A 34-year-old post-partum female having dermatomyositis developed headache and became comatose after a seizure episode. Magnetic resonance imaging of brain showed a massive left ganglio-capsular bleed for which decompressive surgery was done. Computed tomographic angiography showed multiple foci of narrowing and irregularities in distal cerebral vessels. In view of dermatomyositis, the diagnosis of vasculitis was considered and pulse therapy of intravenous methylprednisolone was started. The patient, however, showed no improvement and developed new brain infarcts. She was subsequently taken up for a diagnostic cerebral angiography which showed multifocal severe narrowing in bilateral major cerebral arteries. These angiographic abnormalities showed excellent reversibility to intra-arterial milrinone and hence, reversible cerebral vasoconstriction syndrome (RCVS) was diagnosed. Normal angiographic findings in the first week do not rule out the disease and a repeat angiography should be considered if the clinical suspicion of the RCVS is high. Intra-arterial milrinone has a high diagnostic utility.


2021 ◽  
Author(s):  
Ana Luísa Lopes Espínola da Costa Reis ◽  
Leonardo Henrique Gandolfi de Souza ◽  
Vitor Roberto Pugliesi Marques

Introduction: The ischemic stroke is one of the main causes of death and disability in Brazil. Among the main risk factors are age, atrial fibrillation (AF), diabetes, dyslipidemia and physical inactivity. The main etiology of stroke is cardioembolic, resulting in obstruction of the cerebral arteries by a thrombus of cardiac origin. The artery most affected in ischemic strokes is the middle cerebral artery. The stroke has main characteristics, with emphasis on the sudden onset of symptoms, involvement of a focal area, ischemia caused by obstruction of a vessel and neurological deficits depending on the affected area. Graphesthesia is defined as a cutaneous sensory ability to recognize letters or numbers traced on the skin. The loss of this sensory ability is known as agraphesthesia. Case Report: M.A.F.O. female, 78a, arrived at the UPA complaining of mental confusion. Patient denies previous stroke. Personal history of systemic arterial hypertension. Upon physical examination, the patient was conscious, self and disoriented and inattentive. He was able to repeat and evoke words, without measurable motor déficits. Left upper limb with agraphestesia. Computed tomography was requested, which showed an extensive hypodense area in the right parietoccipital region, which leads to the erasure of the furrows between the adjacent gyres, which may correspond to a recent ischemic event. Magnetic resonance imaging, diffusion-restricted area with correspondence on the ADC map, located in the right temporoparietal region inferring an acute ischemic event. An electrocardiogram was also requested, which showed an irregular rhythm, characteristic of atrial fibrillation, resulting in a diagnostic hypothesis of cardioembolic ischemic stroke. Discussion: The involvement of post-central ischemic gyrus lesions may correspond to paresthesia, anesthesia, hypoesthesia; the involvement of secondary and terciary areas of sensitivity in the upper parietal lobe, especially in the active movements of the hand and in the modalities of integrated sensitivity, their lesions may be clinically affected by: apraxias, dysgraphias, hemineglect, agraphestesia, stereoagnosia and spacial disorientation.


2019 ◽  
Vol 12 (12) ◽  
pp. e230606 ◽  
Author(s):  
Szilard Laszlo Safran ◽  
Christian Balmer ◽  
Georges Savoldelli

We describe the case of a 25-year-old parturient who presented sudden onset and short-lived severe headache caused by reversible cerebral vasoconstriction syndrome (RCVS) during an emergency caesarean section. The syndrome was triggered by phenylephrine administered intravenously to correct arterial hypotension following spinal anaesthesia. RCVS is a clinical and radiological syndrome attributed to transient disturbance in the control of cerebral arterial tone resulting in vasospasms. The syndrome can be precipitated by several triggers, including vasoactive drugs, often used during spinal anaesthesia, illicit drugs, pregnancy and postpartum state. Diagnosis and management can be challenging during pregnancy, peripartum or post partum, since many medications commonly used during these periods must be avoided to prevent triggering RCVS. The aim of this report is to raise the awareness, particularly for anaesthesiologists and obstetricians, of this rare and potentially serious syndrome. We discuss diagnosis, triggers, pathogenesis, clinical course and complications, as well as coordinated multidisciplinary management plans.


BMJ Open ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. e041776
Author(s):  
Ryan Daniel Gotesman ◽  
Naomi Niznick ◽  
Brian Dewar ◽  
Dean A Fergusson ◽  
Risa Shorr ◽  
...  

IntroductionReversible cerebral vasoconstriction syndrome (RCVS) is characterised by severe, recurrent thunderclap headaches (TCHs) and vasoconstriction of cerebral arteries that resolve within 3 months. Abnormalities on non-contrast CT (NCCT) such as ischaemic strokes, intracerebral haemorrhage and subarachnoid haemorrhages are frequently observed on brain imaging of patients with RCVS though their prevalence varies considerably between studies. The aim of this systematic review and meta-analysis is to estimate the prevalence of NCCT abnormalities seen on neuroimaging of adult patients with RCVS.Methods and analysisWe will search the Medline, Embase and the Cochrane Library databases for studies on the prevalence of NCCT abnormalities on neuroimaging of patients with RCVS. Search results will be screened for eligibility by title and abstract. Suitable studies will be fully reviewed and relevant data extracted using a data abstraction form. The studies will be assessed for methodological quality, risk of bias and heterogeneity. Prevalence estimates across studies will be pooled using a random-effects model and subgroup analysis will be performed to assess the impact of age, sex, publication year and study design on prevalence of vascular lesions. Sensitivity analysis will be used to investigate the robustness of the findings. This protocol has been devised using the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols 2015 checklist.Ethics and disseminationFormal ethics is not required as primary data will not be collected. The findings of this study will be disseminated through a peer-reviewed publication and conference presentations.Trial registration numberCRD42020190637.


Sign in / Sign up

Export Citation Format

Share Document